Eli changes the understanding of what is possible, doctor says

Lesli Bales-Sherrod

4:08 PM, Nov 2, 2013

Special to the News Sentinel
“Eli being silly at Vandy today. We had a great, but very long busy day,” says his mom Dawn James on her community Facebook page Baby Elijah James. “There’s a brand new medical carseat out. They can actually do the cutout for his spine in the carseat so he will no longer have any pressure on the area that is breaking down.”

Though Spina Bifida Awareness Month just ended three days ago, there is little published information on rachischisis, the severe form of spina bifida that Elijah James was diagnosed with at 16 weeks gestation.

"There are a few scattered cases of rachischisis survivors in medical literature, but very few," explains neonatologist J. Thomas Meadows Jr, M.D., of University of Tennessee Medical Center. "Rachischisis is a name given to the most extreme forms of spina bifida or spinal dysraphism."

Spina bifida simply means the spine is open at some point while rachischisis is a term given when a large portion of the spine is open.

"Thus, while rachischisis survival isn't impossible, it is highly improbable due to many issues," Dr. Meadows notes. "It really is amazing that Eli is with us today."

Dr. Meadows met Will and Dawn James during prenatal counseling for Eli and was one of his primary providers when Eli was born, seeing him almost daily. Although Eli is no longer his patient, the family keeps in touch with Dr. Meadows, who remains interested in his development.

"A quick (medical records) search shows between five and six case reports, but we have to understand that there must be other cases out there that simply aren't reported," Dr. Meadows says. "As you can see from the troubling images Google pops up when you search for rachischisis, many cases are aborted by the mother's body automatically. Her body knows something isn't going correct with the pregnancy and attempts to end it. Either way, Eli's case is very rare."

Overall survival is rare due to many complications, Dr. Meadows explains. Often the brainstem of the patient is affected, and newborns simply have no respiratory drive. An open spine is a high risk for sepsis/meningitis, which could be fatal, and severe hydrocephalus could lead to apnea.

"Eli's brainstem is preserved, which is why he was able to breathe independently (at birth)," Dr. Meadows explains. "However, the hydrocephalus and open spine did cause a significant number of apnea events and near cardiac arrest while he was admitted (to the hospital). I recall intubating him many times and nearly performing chest compressions. Dawn herself has performed CPR on Eli many times. Thank God with his spinal decompression (surgery) this has all resolved."

Another important surgery for Eli placed a VP shunt to relieve the high pressure in his brain, which was very compressed at birth.

"After the pressure was relieved, his brain expanded and recovered quite well," Dr. Meadows remembers. "The miracle of babies is that if you relieve the pressure from the hydrocephalus, the tissue will expand and can recover."

Eli lives with 27 different diagnoses, which Dawn lists on the About section of the Baby Elijah James public Facebook page. He has had 17 surgeries for procedures ranging from spinal closure to bowel and bladder procedures. His last surgery on October 3 was to remove his tonsils and adenoids as well as tissue from the inside of his nostrils to make his nasal passages larger, all in an effort to open up his airway and increase his oxygen levels, which could help his heart and reverse his pulmonary hypertension.

Still, Eli can do a lot of things doctors never expected.

"He has obvious developmental delay, but I'm very impressed with his progression," Dr. Meadows says. "I see them upon return visits, and Eli continues to astound me. I never thought he'd make the advancements he has."

That's why Dr. Meadows is working on sharing Eli's experiences with the medical community: to raise awareness of his condition and offer families an understanding of what is possible.

"It should always be understood, however, that such a severe diagnosis is not to be taken lightly, and the risk of not surviving is great," Dr. Meadows cautions. "For these reasons, we strive to educate and understand each family unit so they can make the best possible choices for their newborn and their family."